The Churg-Strauss syndrome

Abstract

The Churg-Strauss syndrome is a small vessel vasculitis associated with asthma and eosinophilia. It can sometimes present in unusual ways such as mononeuritis multiplex or subarachnoid hemorrhage. The various symptoms and signs are discussed, such as fever, skin lesions and allergic rhinitis. The sudden onset of blindness and deafness can occur. Cardiac involvement is common, but central nervous system lesions are rare. The diagnosis of the syndrome is based on clinical findings such as asthma, eosinophilia, neuropathy, and sinusitis, and it can certainly mimic conditions such as the Guillain-Barr´ e syndrome. The cause of the syndrome is uncertain, but studies have suggested that it may well be an autoimmune disease. However, an allergy may be only one of several mechanisms triggering symptoms. There seems to be no doubt that certain drugs, notably leukotriene-receptor antagonists, can be to blame, and so can eotoxin-3. Symptoms can respond to treatment with immunosupressive drugs, and since their introduction, the survival rate has been markedly increased. Trials with other drugs, such as rituximab and omalizumab are reported. In cases of intractable asthma, the diagnosis of this syndrome should be considered.